[CIS-PAGID] (no subject)

[christian.wysocki at yale.edu]

Thanks very much for your replies (everyone!).

We did take a look at Tregs via CD25 vs CD127 staining in the past, and found
them to be similar in propotion to her mother (about 5%).  I don't 
think we did
FoxP3 staining, I'll have to find out, may be worth another look.

Quoting "Christine M. Seroogy, MD" <cmseroogy at pediatrics.wisc.edu>:


> Dear Christian,

>

> Have you looked at Treg cells in your patient?

>

> See the following report, your patient has some similar features:

>

> http://www.sciencedirect.com/science/article/pii/S1081120610602517

>

> Chris

>

> Christine M. Seroogy MD, FAAAAI

> Associate Professor

> University of Wisconsin

> Department of Pediatrics

> Division of Allergy, Immunology & Rheumatology

> 1111 Highland Avenue

> 4139 WIMR

> Madison, WI  53705-2275

> phone: 608-263-2652

> fax: 608-265-9721

>

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>

> On 2/22/12 4:47 PM, "christian.wysocki at yale.edu"

> <christian.wysocki at yale.edu> wrote:

>

>> We have a really sick young lady, 24 years old with CVID, enteropathy and

>> other

>> autoimmune sequelae.  Her enteropathy has been severe for many years and

>> refractory to numerous immunomodulatory therapies as detailed below.  She

>> has

>> required TPN since september 2011.  We are hoping to get opinions as to

>> any

>> therapies for severe, refractory CVID associated enteropathy, that people

>> have

>> had success with...we are particularly interested in Rituximab, and

>> wondered

>> what peoples experience with it were, but are open to any and all

>> suggestions.

>> Please see a more detailed clinical description below.

>>

>> Severe chronic diarrhea since age 3.  Biopsies in the past showed a

>> celiac-like

>> process with villous blunting in the small bowel and lymphocytic

>> infiltrates

>> (primarily CD3+....don't know CD4 or 8) in small and large bowel.  She

>> failed a

>> gluten-free diet, and was somewhat improved on prednisone and Imuran.

>>

>> In adolescence, she developed a bad pneumonia, and recurrent sinusitis,

>> and was

>> referred to us when total IgG, A and M were found to be undetectable

>> (total IgG

>> had been 1500, IgA <7 when tested at age 3).  In the meantime, she had

>> developed type 1 diabetes and JRA.  She has been on Ig replacement

>> since...made

>> more difficult by GI protein loss causing difficulty maintaining troughs.

>> She

>> is now on Hizentra and doing better from that perspective. We have done

>> numerous flow analyses over the years....initially B total B cells were

>> quite

>> low (27/ul), but those have come up to normal more recently.  We did a

>> detailed

>> analysis 2 years ago, which showed almost no class switched memory B

>> cells,

>> normal CD21.

>>

>> The GI process still rages out of control, such that she has been on TPN

>> since

>> September.  The GI docs have tried numerous immunomodulatory agents over

>> the

>> years which seem to provide only transient, if any, benefit, including

>> oral

>> steroids (which she is now on chronically), Imuran, cyclosporin, Remicade,

>> Humira and most recently, Cimzia.  A 6 month trial of Cimzia has failed.

>>

>> Thanks for any help or advice you can provide,

>> - Chris Wysocki, Yale Allergy and Immunology fellow

>